Quality of life after cardiac surgery

The aim of this study was to determine a health-related quality of life two or more years after a cardiac surgery using the MOS-36 item short-form health survey questionnaire. 330 members of "Croatian society of patients who underwent cardiac surgery" were asked and 196 responders, 122 men and 74 women, were included in survey, all of them more than two years after surgery and older than fifty years of age. The answers were collected by phone. Women gained better results for Physical Functioning (p < 0.003) and men estimated better scores for psychical health (p < 0.001). Our patients gained some different results in respect to the Croatian sample of healthy people of the same age. They declared better General Health (p < 0.001), less Bodily Pain (p < 0.001), but inferior Role-Emotion (p < 0.001) and Social Functioning (p < 0.001). The estimations of QOL improving were comparable with the results of numerous shorter follow-ups.     

The quality of life after cardiac surgery.

A follow-up study to assess the quality of life after cardiac surgery was begun in 1973. The results, for a total of 383 patients followed up for a maximum of 44 months after surgery, showed an overall improvement in all aspects of life for most patients.     

Mouse models of cardiac chamber formation and congenital heart disease

Congenital heart defects are among the most prevalent and serious birth anomalies in humans. These defects usually involve abnormalities in the formation of specific structures of the developing heart, such as individual chambers or the structures separating one chamber from another. The molecular and cellular pathways responsible for normal heart formation are not well understood, but recent data on single gene knockouts has begun to shed light on the crucial processes that when disrupted result in congenital heart defects.     

Sudden unexpected death in children with congenital heart disease.

Of 18,000 children with organic heart disease evaluated at The Hospital for Sick Children, Toronto between 1940 and 1971, 33 died suddenly and unexpectedly between 1 and 21 years of age. Nine had discrete obstruction of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract. Pulmonary vascular disease caused seven sudden deaths, and arrhythmias (usually due to atrioventricular block) caused seven more. Of the five other children who died suddenly three had transposition of the great arteries, one had a complex cyanotic heart defect and one had an anomalous course of the left coronary artery, which originated from the right sinus of Valsalva. With earlier investigation of aortic stenosis, earlier closure of ventricular septal defect to avoid pulmonary vascular disease, better design of artificial pacemakers and better investigation of patients with angina, many of these deaths will be avoided in the future.     

Myostatin is elevated in congenital heart disease and after mechanical unloading

Background

Myostatin is a negative regulator of skeletal muscle mass whose activity is upregulated in adult heart failure (HF); however, its role in congenital heart disease (CHD) is unknown.

Methods

We studied myostatin and IGF-1 expression via Western blot in cardiac tissue at varying degrees of myocardial dysfunction and after biventricular support in CHD by collecting myocardial biopsies from four patient cohorts: A) adult subjects with no known cardiopulmonary disease (left ventricle, LV), (Adult Normal), (n = 5); B) pediatric subjects undergoing congenital cardiac surgery with normal RV size and function (right ventricular outflow tract, RVOT), (n = 3); C) pediatric subjects with worsening but hemodynamically stable LV failure [LV and right ventricle (LV, RV,)] with biopsy collected at the time of orthotopic heart transplant (OHT), (n = 7); and D) pediatric subjects with decompensated bi-ventricular failure on BiVAD support with biopsy collected at OHT (LV, RV, BiVAD), (n = 3).

Results

The duration of HF was longest in OHT patients compared to BIVAD. The duration of BiVAD support was 4.3±1.9 days. Myostatin expression was significantly increased in LV-OHT compared to RV-OHT and RVOT, and was increased more than double in decompensated biventricular HF (BiVAD) compared to both OHT and RVOT. An increased myostatin/IGF-1 ratio was associated with ventricular dysfunction.

Conclusions

Myostatin expression in increased in CHD, and the myostatin/IGF-1 ratio increases as ventricular function deteriorates. Future investigation is necessary to determine if restoration of the physiologic myostatin/IGF-1 ratio has therapeutic potential in HF.     

Prenatal screening for congenital heart disease.

Routine ultrasound examination of the fetus is already established in most obstetric units in Britain. A simple method was devised to evaluate one section of the fetal heart systematically. Examination of this section, the four chamber view, may readily be incorporated into routine obstetric screening. Severe cardiac abnormalities detectable in this view occur in two per 1000 pregnancies. For six years the department of paediatric cardiology at Guy''s Hospital, London, served as a referral centre for fetal echocardiography. As teaching became more widespread an increasing proportion of cases of cardiac anomaly were referred because the obstetrician suspected abnormality on examination of the four chamber view. Currently 80% of detected abnormalities are referred for this reason. Further extension and organisation of teaching might result in most severe cardiac malformations being detected in early prenatal life.     

Inheritance of congenital heart disease

Congenital heart defects (CHD) are the most common developmental anomalies and are the leading noninfectious cause of mortality in newborn babies. It has been estimated that between four and ten live-born infants per 1000 have a cardiac malformation (0.4 to 1.0%), 40% of which are diagnosed in the first year of life. The European Registration of Congenital Anomalies (EUROCAT) reported a prevalence of 58.9/10,000 live births in the northern part of the Netherlands (0.6%). Hoffman estimated that the true prevalence of CHD may be as high as 53 per 1000 pregnancies (5.3%), including a 20% occurrence of heart defects in spontaneous abortion, a 10% occurrence in stillbirth, and a 1% occurrence in live birth.     

Selective angiocardiography in congenital heart disease

Angiocardiography has come to be a highly specialized procedure and there are definite indications for doing it. In specific cases it offers clear-cut advantages. Technical improvements permit a single injection of a small amount of contrast substance. Selective angiocardiography is advised where the particular detail of a certain region in the heart is desired.     

Differences between atrial and ventricular protein profiling in children with congenital heart disease

The purpose of the present study was to compare protein profiling of atria and ventricles in children operated for congenital heart disease. Tissue samples were obtained during surgery from patients with normoxemic (ventricular and atrial septal defects) and hypoxemic (tetralogy of Fallot) diseases. Protein fractions were isolated by stepwise extraction from both fight ventricular and atrial musculature. The concentration of total atrial protein in the normoxemic patients exceeded the ventricular value (110±2.1 vs 99.9±4.0mg.g^–1 wet weight, respectively); in the hypoxemic group this atrio-ventricular difference disappeared. The concentration of contractile proteins in all cardiac samples was significantly higher in the ventricles as compared with atria, while the concentration of collagenous proteins was significantly higher in the atria (due to a higher amount of the insoluble collagenous fraction). The concentration of sarcoplasmic proteins (containing predominantly enzyme systems for aerobic and anaerobic substrate utilization), however did not differ between ventricles and atria. Furthermore, ventricular contractile fractions obtained from both normoxemic and hypoxemic patients were contaminated with the myosin light chain of atrial origin. Soluble collagenous fractions (containing newly synthesized collagenous proteins, predominantly collagen I and III), derived from all ventricular samples, were contaminated by low molecular weight fragments (mol. weight 29–35 kDa). The proportion of the soluble collagenous fraction was significantly higher in atrial but not in ventricular myocardium of hypoxemic children as compared with the normoxemic group. It seems, therefore, that lower oxygen saturation affects the svnthesis of collagen preferentially in atrial tissue.     

DNA microarray analysis for human congenital heart disease

Right ventricular hypertrophy and failure are prominent features in cyanotic congenital heart disease, tetralogy of Fallot (TF). Patients with TF require primary cardiac surgery at a very young age. To gain insight into the underlying molecular mechanisms of right ventricular hypertrophy and to identify gene(s) involved in TF, differential gene expression profile was assessed using expression-based microarray technology on right ventricular biopsies from young TF patients who underwent primary correction. By using quantitative immunohistochemistry, expression of vascular endothelial growth factor (VEGF), flk-1, and extracellular matrix (ECM) proteins (collagens and fibronectin) as well as vessel counts and myocyte cell size was evaluated in TF patients in relation to age-matched controls. Among 236 genes showing altered expression pattern in TF patients, VEGF (1.8-fold) and ECM markers were clearly upregulated (fibronectin, 2.4-fold; collagen Iα, 7.5-fold; and collagen III, 4.4-fold); flk-1 and most matrix metalloproteinases (MMPs) remained unchanged, except the levels of MMP-13 and-17 declined. Tissue inhibitors of metalloproteinases showed a downregulated pattern. Staining of VEGF in cardiomyocytes and of ECM proteins (fibronectin, collagen I and III) in interstitial as well as in perivascular area was increased (p<0.01) in TF patients. Morphometric analysis revealed enhanced vascular density (p<0.05) with unchanged wall thickness and enlarged myocyte cross-sectional areas (p<0.01) with linear correlation (p<0.01) with the age in TF-1 patients. We conclude that the upregulation of genes encoding VEGF and ECM proteins are the key events contributing to right ventricular hypertrophy and stunted angiogenesis in patients with TF.